中华检验医学杂志
中華檢驗醫學雜誌
중화검험의학잡지
CHINESE JOURNAL OF LABORATORY MEDICINE
2012年
5期
418-422
,共5页
张永良%汪伟山%周玉球%肖奇志%谢建红
張永良%汪偉山%週玉毬%肖奇誌%謝建紅
장영량%왕위산%주옥구%초기지%사건홍
α地中海贫血%红细胞指数%基因型
α地中海貧血%紅細胞指數%基因型
α지중해빈혈%홍세포지수%기인형
alpha-Thalassemia%Erythrocyte indices%Genotype
目的 探讨α-地中海贫血(α-地贫)基因型和红细胞参数之间的关系.方法 选取2006年1月至2010年6月珠海市妇幼保健院337例成年α-地贫基因携带者,所有病例均排除缺铁、α-地贫双重杂合子和纯合子、α复合β-地贫和异常血红蛋白病.以α珠蛋白基因型不同分为3组:(1)-/αα或αTα/αα型:静止型地贫组(ST组)83例；(2)--SEA/αα型:标准型地贫组(TT组)210例；(3)-α/--SEA或αTα/--SEA型:中间型地贫组(IT组)或HbH病组44例.选取同期154名健康体检者做为对照组(NC组).回顾性分析各组红细胞参数,包括RBC、Hb、MCV、MCH、MCHC和RDW-CV,采用方差分析及SNK检验比较上述各组间红细胞参数的差异.结果 除Hb F外,各组间红细胞参数差异均有统计学意义.MCV和MCH从高到低依次为NC组[(86.6±5.2)fl、(29.5±2.1)pg] ＞ST组[(80.1±3.3) fl、(26.7±1.3)pg]＞TT组[(68.5±3.4)fl、(22.0±1.2)pg]＞IT组[(66.6± 7.1)fl、(20.0±2.2)pg],差异均有统计学意义(F=580.67、761.19,P均＜0.05).而反映RBC大小异质性的RDW-CV则是IT组(22.3±3.4)％＞TT组(14.9±1.2)％＞ST组(13.8±1.6)％＞NC组(13.2±1.4)％,差异有统计学意义(F=347.25,P＜0.05).在ST组中,-α3.7/αα亚组的MCHC为( 335.6±8.0) g/L,高于-α4.2/αα亚组的(330.0±7.2)g/L和αTα/αα亚组的(328.4±9.5) g/L,差异有统计学意义(F=6.07,P＜0.05).在IT组中,αTα/--SEA亚组的MCV为(70.1±7.2)fl,高于-α3.7/--SEA亚组的(63.4±5.9)fl和-α4.2/--SEA亚组的(64.1±4.0)fl；而αTα/--SEA亚组的MCHC为(289.7±21.2)g/L,低于其他2个亚组的(306.3±8.4)、(306.1±8.7) g/L,差异均有统计学意义(F=5.55、8.72,P均＜0.05).除Hb A2和Hb F外,α珠蛋白基因缺陷的个数与RBC计数和RDW-CV呈正相关(r=0.318、0.580,P均＜0.01),而与其他RBC指标则呈负相关(r=-0.483、-0.827、-0.744、-0.684,P均＜0.01).结论 α-地贫的贫血程度与缺陷的α珠蛋白基因个数密切相关,并以Hb降低和RBC升高为主要特征.非缺失型Hb H病(αTα/--SEA)的贫血程度比缺失型Hb H病严重,-α4.2/--SEA型Hh H病义重于-α3.7/--SEA型Hb H病.
目的 探討α-地中海貧血(α-地貧)基因型和紅細胞參數之間的關繫.方法 選取2006年1月至2010年6月珠海市婦幼保健院337例成年α-地貧基因攜帶者,所有病例均排除缺鐵、α-地貧雙重雜閤子和純閤子、α複閤β-地貧和異常血紅蛋白病.以α珠蛋白基因型不同分為3組:(1)-/αα或αTα/αα型:靜止型地貧組(ST組)83例；(2)--SEA/αα型:標準型地貧組(TT組)210例；(3)-α/--SEA或αTα/--SEA型:中間型地貧組(IT組)或HbH病組44例.選取同期154名健康體檢者做為對照組(NC組).迴顧性分析各組紅細胞參數,包括RBC、Hb、MCV、MCH、MCHC和RDW-CV,採用方差分析及SNK檢驗比較上述各組間紅細胞參數的差異.結果 除Hb F外,各組間紅細胞參數差異均有統計學意義.MCV和MCH從高到低依次為NC組[(86.6±5.2)fl、(29.5±2.1)pg] ＞ST組[(80.1±3.3) fl、(26.7±1.3)pg]＞TT組[(68.5±3.4)fl、(22.0±1.2)pg]＞IT組[(66.6± 7.1)fl、(20.0±2.2)pg],差異均有統計學意義(F=580.67、761.19,P均＜0.05).而反映RBC大小異質性的RDW-CV則是IT組(22.3±3.4)％＞TT組(14.9±1.2)％＞ST組(13.8±1.6)％＞NC組(13.2±1.4)％,差異有統計學意義(F=347.25,P＜0.05).在ST組中,-α3.7/αα亞組的MCHC為( 335.6±8.0) g/L,高于-α4.2/αα亞組的(330.0±7.2)g/L和αTα/αα亞組的(328.4±9.5) g/L,差異有統計學意義(F=6.07,P＜0.05).在IT組中,αTα/--SEA亞組的MCV為(70.1±7.2)fl,高于-α3.7/--SEA亞組的(63.4±5.9)fl和-α4.2/--SEA亞組的(64.1±4.0)fl；而αTα/--SEA亞組的MCHC為(289.7±21.2)g/L,低于其他2箇亞組的(306.3±8.4)、(306.1±8.7) g/L,差異均有統計學意義(F=5.55、8.72,P均＜0.05).除Hb A2和Hb F外,α珠蛋白基因缺陷的箇數與RBC計數和RDW-CV呈正相關(r=0.318、0.580,P均＜0.01),而與其他RBC指標則呈負相關(r=-0.483、-0.827、-0.744、-0.684,P均＜0.01).結論 α-地貧的貧血程度與缺陷的α珠蛋白基因箇數密切相關,併以Hb降低和RBC升高為主要特徵.非缺失型Hb H病(αTα/--SEA)的貧血程度比缺失型Hb H病嚴重,-α4.2/--SEA型Hh H病義重于-α3.7/--SEA型Hb H病.
목적 탐토α-지중해빈혈(α-지빈)기인형화홍세포삼수지간적관계.방법 선취2006년1월지2010년6월주해시부유보건원337례성년α-지빈기인휴대자,소유병례균배제결철、α-지빈쌍중잡합자화순합자、α복합β-지빈화이상혈홍단백병.이α주단백기인형불동분위3조:(1)-/αα혹αTα/αα형:정지형지빈조(ST조)83례；(2)--SEA/αα형:표준형지빈조(TT조)210례；(3)-α/--SEA혹αTα/--SEA형:중간형지빈조(IT조)혹HbH병조44례.선취동기154명건강체검자주위대조조(NC조).회고성분석각조홍세포삼수,포괄RBC、Hb、MCV、MCH、MCHC화RDW-CV,채용방차분석급SNK검험비교상술각조간홍세포삼수적차이.결과 제Hb F외,각조간홍세포삼수차이균유통계학의의.MCV화MCH종고도저의차위NC조[(86.6±5.2)fl、(29.5±2.1)pg] ＞ST조[(80.1±3.3) fl、(26.7±1.3)pg]＞TT조[(68.5±3.4)fl、(22.0±1.2)pg]＞IT조[(66.6± 7.1)fl、(20.0±2.2)pg],차이균유통계학의의(F=580.67、761.19,P균＜0.05).이반영RBC대소이질성적RDW-CV칙시IT조(22.3±3.4)％＞TT조(14.9±1.2)％＞ST조(13.8±1.6)％＞NC조(13.2±1.4)％,차이유통계학의의(F=347.25,P＜0.05).재ST조중,-α3.7/αα아조적MCHC위( 335.6±8.0) g/L,고우-α4.2/αα아조적(330.0±7.2)g/L화αTα/αα아조적(328.4±9.5) g/L,차이유통계학의의(F=6.07,P＜0.05).재IT조중,αTα/--SEA아조적MCV위(70.1±7.2)fl,고우-α3.7/--SEA아조적(63.4±5.9)fl화-α4.2/--SEA아조적(64.1±4.0)fl；이αTα/--SEA아조적MCHC위(289.7±21.2)g/L,저우기타2개아조적(306.3±8.4)、(306.1±8.7) g/L,차이균유통계학의의(F=5.55、8.72,P균＜0.05).제Hb A2화Hb F외,α주단백기인결함적개수여RBC계수화RDW-CV정정상관(r=0.318、0.580,P균＜0.01),이여기타RBC지표칙정부상관(r=-0.483、-0.827、-0.744、-0.684,P균＜0.01).결론 α-지빈적빈혈정도여결함적α주단백기인개수밀절상관,병이Hb강저화RBC승고위주요특정.비결실형Hb H병(αTα/--SEA)적빈혈정도비결실형Hb H병엄중,-α4.2/--SEA형Hh H병의중우-α3.7/--SEA형Hb H병.
Objective To investigate the correlation between the erythrocyte indices and the genotypes of alpha thalassemia.Methods 337 carriers with various genotypes of alpha-thalassaemia ( iron deficiency,alpha-thalassemia double heterozygote and homozygote,α-compounding β-thalassemia and abnormal hemoglobinopathy were excluded) were classified into three groups based on different genotypes of alpha-thalassaemia including silent thalassemia group (ST,83 cases),α-thalassemia trait group (TT,210cases) and intermediate thalassemia group( IT,44 cases),and 154 healthy adults were randomly choosed as normal control The erythrocyte indices involving in RBC,Hb,MCV,MCH,MCHC and RDW-CV were retrospectively analyzed and the difference of which was compared by analysis of variance and SNK test among aboved-mentioned groups.Results There were statistical significance among groups about erythrocyte indices except Hb F.The order of the level of MCV and MCH was NC [( 86.6 ± 5.2) fl,( 29.5 ± 2.1 ) pg] ＞ST[(80.1 ±3.3) fl,(26.7±1.3) pg] ＞TT[(68.5 ±3.4) fl,(22.0 ±1.2) pg] ＞IT[(66.6±7.1)fl,(20.0 ±2.2) pg,F =580.67,761.19,P ＜0.05].And the size of RDW-CV was IT(22.3 ±3.4)％ ＞TT (14.9±1.2) ％ ＞ST(13.8±1.6)％ ＞NC(13.2±1.4)％(F=347.25,P＜0.05).In ST group,the value of MCHC of -α3.7/αα subgroup( 335.6 ± 8.0) g/L was higher than that of -α4.2/αα subgroup( 330 ±7.2) g/L and αTα/αα subgroup (328.4 ±9.5) g/L(F=6.07,P ＜0.05).Meanwhile,in IT group,the value of MCV of αTα/--SEA subgroup( 70.1 ± 7.2 ) fl was higher than that of -α3.7/--SEA subgroup ( 63.4 ±5.9) fl and -α4.2/--SEA subgroup ( 64.1 ± 4.0 ) fl ( F =5.55,P ＜ 0.05 ).However,the value of MCHC of αTα/--SEA subgroup( 289.7 ± 21.2 ) g/L was lower than that of other two subgroups [( 306.3 ± 8.4 ),(306.1 ± 8.7) g/L,F =8.72,P ＜0.05].Except Hb A2 and Hb F,there was positive correlation between the number of deleted α-globin gene and that of RBC and RDW-CV ( r =0.318 and 0.580,P ＜0.01 ).Nevertheless,there was negative correlation between the number of deleted α-globin gene and that of the other erythrocyte indices (r =-0.483,-0.827,-0.744 and -0.684,P all ＜0.01 ).Conclusions There is close correlation between the degree of anemia and the number of deleted α-globin gene characterized by Hb reduction and RBC increasing.In addition,the anemia degree of non-deletional Hb H disease is severer than that of deletional Hb H,which of Hb H disease with -α4.2/--SEA is severer than that with -α3.7/--SEA.
